Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

1.3.3.6

Official Name

acyl-CoA oxidase

Name from literature

acyl CoA oxidase

Pathway from literature

very-long-chain fatty acid peroxisomal oxidation

Pathway from KEGG

Lipid Metabolism; Fatty acid metabolism; map00071

Cellular Processes; Endocrine System; PPAR signaling pathway; map03320

Lipid Metabolism; Biosynthesis of unsaturated fatty acids; map01040

Lipid Metabolism; alpha-Linolenic acid metabolism; map00592

Organisms

Human (9606)

Genome localization

17q24-q25|17q25.1[51 ], 4p15.3[8310 ],

Comments

A flavoprotein (FAD). Acts on CoA derivatives of fatty acids with chain lengths from 8 to 18.

Rate-limiting Description

"In the present study, the cloning, expression and characterization of the rate-limiting enzyme of the peroxisomal beta-oxidation spiral, acyl CoA oxidase (AOX), from koala (Phascolarctos cinereus) liver is described." (12758125)

Regulatory Information

Upstream transcription factor

5465

Regulatory type

Detail

phosphorylation;

"Global, in vivo, and site-specific phosphorylation dynamics in signaling networks." (17081983)

regulatory enzyme;

"As revealed by Northern and Western blotting there was marked elevation of the mRNA (190%) and protein (180%) of the peroxisomal branched-chain acyl-CoA oxidase. This protein is the key regulatory enzyme for the side chain oxidation of cholesterol for bile acid synthesis, a pathway associated with mature hepatocytes. " (9921653)

phosphorylation;

Q15067

phosphorylation;

Q15067:from_uniprot:26_Phosphoserine

phosphorylation;

Q15067:from_uniprot:310_Phosphoserine

phosphorylation;

Q15067:from_uniprot:649_Phosphoserine

transcriptional factor;PPARalpha(5465)

"Differential regulation of the cynomolgus, human, and rat acyl-CoA oxidase promoters by PPARalpha." (16828988)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [O15254, Q15067 ];
GO:0006693 (P) prostaglandin metabolic process [Q15067 ];
GO:0006635 (P) fatty acid beta-oxidation [O15254, Q15067 ];
GO:0003995 (F) acyl-CoA dehydrogenase activity [O15254, Q15067 ];
GO:0008206 (P) bile acid metabolic process [O15254 ];
GO:0009055 (F) electron carrier activity [O15254, Q15067 ];
GO:0005777 (C) peroxisome [O15254 ];
GO:0006091 (P) generation of precursor metabolites and energy [Q15067 ];
GO:0055114 (P) oxidation reduction [O15254, Q15067 ];
GO:0003997 (F) acyl-CoA oxidase activity [O15254, Q15067 ];

Subcellular localization

Localization

peroxisome [O15254, Q15067 ];

Disease relevance

Disease

Defects in ACOX1 are the cause of pseudoneonatal adrenoleukodystrophy [MIM:264470];
also known as peroxisomal acyl-CoA oxidase deficiency. Pseudo-NALD is a peroxisomal single-enzyme disorder. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty cids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning [Q15067 ];

Links

SwissProt

O15254; Q15067

Entrez Gene

51; 8310

HPRD

02030; 04552



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min